Otorhinolaryngology Unit, Division of Head and Neck Department, IRCCS San Raffaele Scientific Institute, 20132 Milan, Italy.
School of Medicine, Vita-Salute San Raffaele University, 20132 Milan, Italy.
Int J Environ Res Public Health. 2020 Nov 5;17(21):8179. doi: 10.3390/ijerph17218179.
A series of destructive and tumefactive lesions of the oral cavity are increasingly recognized as part of the IgG4-related disease (IgG4-RD) spectrum. We herein examined the clinical, serological, radiological, and histological features of a series of patients referred to our clinic because of oral cavity lesions ultimately attributed to IgG4-RD. In particular, we studied 6 consecutive patients out of 200 patients referred to the immunology outpatient unit who presented with erosive and/or tumefactive lesions of the oral cavity. All patients underwent serum IgG4 measurement, nasal endoscopy, radiological studies, and histological evaluation of tissue specimens. The histological studies included immunostaining studies to assess the number of IgG4+ plasma cells/High-Power Field (HPF) for calculation of the IgG4+/IgG+ plasma cell ratio. Six patients (3% of the entire cohort) were diagnosed with IgG4-RD of the oral cavity based on histological evaluation. A major complaint at presentation was oral discomfort due to bulging mass. A mild to no increase in serum IgG4 was observed. Different patterns of organ involvement were associated with oral lesions. Five patients were treated with immunosuppressive therapy and two patients promptly responded to B-cell depletion with rituximab. Watchful waiting was decided in one patient with no major clinical symptoms. Involvement of the oral cavity is an infrequent manifestation of IgG4-RD but should be taken into consideration as a possible differential diagnosis of tumefactive or erosive lesions once neoplastic conditions are excluded. A histological examination of biopsy samples from the oral cavity represents the mainstay for diagnosis of IgG4-RD.
口腔内一系列破坏性和肿胀性病变越来越被认为是 IgG4 相关疾病（IgG4-RD）谱的一部分。我们在此检查了因口腔病变而最终归因于 IgG4-RD 的一系列患者的临床、血清学、影像学和组织学特征。特别是，我们研究了 200 名免疫门诊患者中连续 6 名出现口腔侵蚀性和/或肿胀性病变的患者。所有患者均接受了血清 IgG4 测量、鼻内窥镜检查、影像学研究和组织学评估。组织学研究包括免疫染色研究，以评估 IgG4+浆细胞/高倍镜视野（HPF）的数量，用于计算 IgG4+/IgG+浆细胞比值。根据组织学评估，6 名患者（整个队列的 3%）被诊断为口腔 IgG4-RD。主要的就诊症状是由于肿块突出导致的口腔不适。观察到血清 IgG4 轻度至中度升高。不同模式的器官受累与口腔病变相关。5 名患者接受了免疫抑制治疗，2 名患者接受利妥昔单抗 B 细胞耗竭治疗后迅速反应。一名无主要临床症状的患者决定密切观察。口腔受累是 IgG4-RD 的罕见表现，但在排除肿瘤性疾病后，应将其视为肿胀性或侵蚀性病变的可能鉴别诊断之一。口腔活检样本的组织学检查是诊断 IgG4-RD 的主要依据。
